Measuring enzyme activity would have comprised several individual experiments, as corticosterone might inhibit different enzymes, furthermore a potential shortage of common co-factors would remain undetected.
Moreover, our data clearly demonstrate that an altered lipid metabolism, as a result of co-enzyme A shortage, could represent one of the underlining causes of the disease.
Recently, we observed a reduction of the bile acids tauro and glycol cholate (Leoni et al., 2012) and an alteration of lipid metabolism in plasma derived from patients with PKAN, likely because of co-enzyme A shortage causing, among others, dysfunctional fat assimilation.
The concentration of cytosolic acetyl-CoA in the livers of KO and WT mice was similar, suggesting that the impact on fat and cholesterol production in the KO mice was primarily due to down regulation of key synthetic enzymes, not to a shortage of the primary building blocks.
His discoveries helped unravel the biology of Tay-Sachs disease and more than two dozen other genetic diseases in which a shortage of lysosomal enzymes causes waste to accumulate in cells and eventually destroy them.
This leads to a shortage in lysosomal enzymes, resulting in a decrease in degradation by this pathway.
In the presence of a shortage of co-enzyme A, this diet could evoke a general impairment of bioenergetic metabolism.
Autophagy-defective cells cannot produce ROS-scavenging enzymes, likely due to a shortage of free amino acids for de novo protein synthesis.
Shortage in substrates or PPDK enzyme leads to maximal activity of PPDK [ 17]).
On the other hand, the germplasms with no colour might have resulted because of inability to hydroxylate the aromatic ring of phenol either due to shortage of electron donor or hydroxylating enzyme (Takahashi and Hamza 1983).
